PULMONARY EMPHYSEMA AND OTHER CARDIORESPIRATORY LESIONS AS PART OF THE MARFAN ABIOTROPHY

PEDIATRICS ◽  
1964 ◽  
Vol 33 (3) ◽  
pp. 356-366
Author(s):  
Robert P. Bolande ◽  
Arthur S. Tucker

Seven cases of Marfan's syndrome are reviewed clinically, radiologically, and pathologically. Six of the seven cases showed evidence of pulmonary dysaeration: (a) Two of the cases showed compression of the left main-stem bronchus by a giant left atrium with atelectasis of the left lung and compensatory emphysema of the right lung. (b) Two of the cases showed evidence of diffuse chronic pulmonary emphysema. Three cases had bilateral apical bullae. (c) One of the cases developed pneumothorax. The lungs of the children with the Marfan syndrome show precocious maturation of the elastic stroma of the alveolar septae. The pathogenesis of emphysema is discussed in relationship to the Marfan abiotrophy of connective tissue.

1988 ◽  
Vol 64 (1) ◽  
pp. 162-173 ◽  
Author(s):  
S. N. Mink ◽  
H. Greville ◽  
A. Gomez ◽  
J. Eng

We examined maximum expiratory flow (Vmax) in two canine preparations in which regional changes in lung mechanical properties were produced. In one experiment serial bronchial obstructions were made to determine whether flow-limiting sites (choke points, CP) would occur in series. With the right lung tied off, constrictions were placed at the left lower lobar bronchus (LLL) and left main-stem bronchus. On deflation from total lung capacity, the obstructed LLL and nonobstructed left upper lobe (LUL) emptied into the obstructed left main-stem bronchus. Although a CP common to both lobes was identified at the main-stem obstruction, which limited total Vmax, we questioned whether there was also a CP at the lobar obstruction that fixed LLL flow. In that case the rate of LLL emptying would not be dependent on the presence of the common (i.e., central) CP and thus the flow contribution of the LUL. We found that when the LUL was removed, the LLL increased its rate of emptying. Thus a lobar CP did not fix LLL flow and CP did not occur in series. In a second experiment emphysema was produced in the left lung to reduce lung recoil, whereas the right lung was normal. CP were identified at approximately lobar bronchi of each lung, and the lungs were emptied at different rates. A CP common to both lungs was not identified. Our results indicate that in localized lung disease, if flows from the different regions are high enough, then wave speed is reached in proximal airways, and a CP occurs centrally rather than peripherally. On the other hand, if flows are low, then wave speed is reached peripherally and a CP common to all lung regions does not occur.


CHEST Journal ◽  
1983 ◽  
Vol 83 (6) ◽  
pp. 928-929 ◽  
Author(s):  
Nelson A Burton ◽  
Stephen M. Fall ◽  
Thomas Lyons ◽  
Geoffrey M. Graeber

ASVIDE ◽  
2021 ◽  
Vol 8 ◽  
pp. 038-038
Author(s):  
Hui Jia ◽  
Wen-Fei Tan ◽  
Hong Ma ◽  
Yong Cui

1990 ◽  
Vol 68 (4) ◽  
pp. 1590-1596 ◽  
Author(s):  
S. B. Oetomo ◽  
J. Lewis ◽  
M. Ikegami ◽  
A. H. Jobe

The effect of exogenous surfactant on endogenous surfactant metabolism was evaluated using a single-lobe treatment strategy to compare effects of treated with untreated lung within the same rabbit. Natural rabbit surfactant, Survanta, or 0.45% NaCl was injected into the left main stem bronchus by use of a Swan-Ganz catheter. Radio-labeled palmitic acid was then given by intravascular injection at two times after surfactant treatment, and the ratios of label incorporation and secretion in the left lower lobe to label incorporation and secretion in the right lung were compared. The treatment procedure resulted in a reasonably uniform surfactant distribution and did not disrupt lobar pulmonary blood flow. Natural rabbit surfactant increased incorporation of palmitate into saturated phosphatidylcholine (Sat PC) approximately 2-fold (P less than 0.01), and secretion of labeled Sat PC increased approximately 2.5-fold in the surfactant-treated left lower lobe relative to the right lung (P less than 0.01). Although Survanta did not alter incorporation, it did increase secretion but not to the same extent as rabbit surfactant (P less than 0.01). Alteration of endogenous surfactant Sat PC metabolism in vivo by surfactant treatments was different from that which would have been predicted by previous in vitro studies.


2018 ◽  
Vol 46 (11) ◽  
pp. 4821-4824
Author(s):  
Mingfeng Yang ◽  
Lan Zhang

Giant left atrium is most commonly associated with rheumatic mitral valve disease, causing a series of cardiac and extracardiac complications. Cardiac complications are often reported, such as atrial fibrillation, decreased cardiac output, and atrial thrombus formation. Extracardiac complications are rarely described in the literature. We report an unusual case of a 55-year-old woman who was diagnosed with rheumatic heart disease 20 years earlier. Her chief complaints were episodes of chest tightness and difficulty breathing, which she had for more than 30 years. Echocardiography showed severe mitral stenosis with severe mitral insufficiency. Contrast-enhanced chest CT showed that the left thoracic cavity was occupied by a giant left atrium. The left main bronchus was compressed, and the left lung showed complete consolidation without pulmonary function.


2019 ◽  
Vol 07 (01) ◽  
pp. e1-e4 ◽  
Author(s):  
Tatjana Tamara König ◽  
Eva Wittenmeier ◽  
Oliver J. Muensterer

Introduction Isolated tracheobronchial injury after blunt trauma of the chest is rare. Because of the high elasticity of the chest in children, they occur mainly in the pediatric population. Case Report We report a case of a 7-year-old girl who experienced complete avulsion of the right main bronchus at the level of the carina after a horse-riding accident. The patient presented with extensive emphysema of the upper chest, neck, and face and severe respiratory distress. Endotracheal intubation led to tension pneumothorax. After insertion of two 17-mm thoracostomy tubes, pneumothorax and a massive air leak persisted. Isolated central bronchial injury was confirmed by computed tomography of the chest. Bronchoscopically guided selective intubation of the left main stem bronchus failed and the patient desaturated, requiring immediate salvage right posterolateral thoracotomy. Simultaneous occlusion of the defect, stabilization, and subsequent selective left lung intubation was possible only after placing a suture at the tracheal rim of the defect for retraction allowing compression of the defect and keeping the lumen open at the same time. Conclusion A cluster of clinical signs with subcutaneous emphysema and refractory pneumothorax with air leak of the thoracotomy tube is indicative of bronchial injury. Endotracheal intubation should be postponed in these cases until after thoracostomy tube placement, if possible. Placing a retraction suture during repair is a maneuver that helps to occlude the defect and keep the remaining tracheobronchial lumen open at the same time to establish crucial ventilation of the contralateral lung.


1993 ◽  
Vol 264 (3) ◽  
pp. R610-R614 ◽  
Author(s):  
J. M. Kisala ◽  
A. Ayala ◽  
R. N. Stephan ◽  
I. H. Chaudry

Although atelectasis frequently occurs after surgery and trauma, and such patients have elevated body temperatures, the mechanism of temperature elevation secondary to atelectasis is unknown. Moreover, a small animal model has not been available to study the pathophysiology of pulmonary atelectasis. The purpose of this study, therefore, was to develop a model of pulmonary atelectasis in rats. Because interleukin-1 (IL-1) and tumor necrosis factor (TNF), both potent pyrogens, are produced by macrophages during infection and inflammation, our aim was also to determine whether alveolar macrophages produce IL-1 or TNF in response to atelectasis. Whole-lung atelectasis was produced in rats by ligating the left main stem bronchus while maintaining ventilation of the right lung. After a 1-h period of atelectasis, alveolar macrophages were harvested from the right and left lungs and incubated for 24 h, and the supernatants were assayed for IL-1 and TNF. Both IL-1 and TNF levels of macrophage cultures from the atelectatic lung were significantly increased compared with the control lung. These results suggest that increased IL-1 or TNF production by alveolar macrophages may be responsible for fever caused by atelectasis.


Author(s):  
Veronika Kroepfl ◽  
Caecilia Ng ◽  
Herbert Maier ◽  
Paolo Lucciarini ◽  
Stefan Scheidl ◽  
...  

Carcinoids of the left main bronchus are rare tumors of the bronchial system and patients often present with dyspnea, asthma-like symptoms, and pneumonia. Gold standard for therapy of carcinoids is surgical resection, but the surgical approach for segmental resection and anastomosis of the left main bronchus is a matter of discussion. With a left-sided approach the access to the bronchus is blocked by the aortic arch and the pulmonary vein. If a right-sided approach is performed, the problem of ventilation during resection and anastomosis of the bronchus occurs. We present a surgical approach from the right side using intraoperative extracorporeal membrane oxygenation to assure oxygen supply for resection of a typical carcinoid of the left main stem bronchus, and discuss the current literature.


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